You are on page 1of 4

G Model

ANORL-731; No. of Pages 4 ARTICLE IN PRESS

European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx

Available online at


International consensus

International consensus (ICON) on treatment of Ménière’s disease

J. Nevoux a,b,∗ , M. Barbara c , J. Dornhoffer d , W. Gibson e , T. Kitahara f , V. Darrouzet g
Department of otology and neurotology, CHU de Bicetre, AP–HP, 94270 Le Kremlin-Bicêtre, France
Saclay university, Paris-Sud Medical School, 94270 Le Kremlin-Bicêtre, France
Department of otology and neurotology, Sapienza university, Rome, Italy
Department of otolaryngology, head and neck surgery, university of Arkansas for medical sciences and Arkansas Children’s Hospital, Little Rock, Arkansas,
Department of otolaryngology, head and neck surgery, university of Sidney, Australia
Department of otolaryngology, Nara medical university, Japan
Department of otolaryngology, Skull Base Surgery, CHU de Bordeaux, université de Bordeaux, 33000 Bordeaux, France

a r t i c l e i n f o a b s t r a c t

Keywords: Objective: To present the international consensus for recommendations for Ménière’s disease (MD) treat-
International consensus ment.
Ménière’s disease Methods: Based on a literature review and report of 4 experts from 4 continents, the recommendations
have been presented during the 21st IFOS congress in Paris, in June 2017 and are presented in this work.
Results: The recommendation is to change the lifestyle, to use the vestibular rehabilitation in the inter-
critic period and to propose psychotherapy. As a conservative medical treatment of first line, the authors
recommend to use diuretics and Betahistine or local pressure therapy. When medical treatment fails,
the recommendation is to use a second line treatment, which consists in the intratympanic injection of
steroids. Then as a third line treatment, depending on the hearing function, could be either the endolym-
phatic sac surgery (when hearing is worth being preserved) or the intratympanic injection of gentamicin
(with higher risks of hearing loss). The very last option is the destructive surgical treatment labyrinthec-
tomy, associated or not to cochlear implantation or vestibular nerve section (when hearing is worth being
preserved), which is the most frequent option.
© 2018 Elsevier Masson SAS. All rights reserved.

1. Introduction algorithm. But this synthesis revealed highly challenging, as among

the countries and/or continents of the participants of this ICON,
Ménière’s disease (MD) treatment must be first based on a only two have drawn a consensus or recommendation applicable
trustable diagnosis. To be confident with the diagnosis, it is rec- in their countries, Japan in 2011 and France in 2016 [3]. Scientific lit-
ommended to use the consensual guidelines and classification of erature was assessed using the Level of Evidence classification (1 to
the AAO-HNS published in 1995 [1] and recently reviewed by the 5) and recommendations were given following the grading of rec-
Equilibrium Committee in 2015 [2]. Although the diagnosis is pri- ommendations assessment, development and evaluation (GRADE)
marily based on the clinical history, clinicians also utilize various scoring system.
tests to confirm the diagnosis before introduction of any treatment. In MD, the aim of the treatment is first to reduce the fre-
The next step is to tailor the treatment for each patient based on quency, and secondarily the severity, of the vertigo crises, with
an algorithm that seems frequently different from one center to a minimal impairment of hearing function, hoping this favor-
another in the same country, even in the same country. During the able result is associated with a hearing and tinnitus improvement
last IFOS Congress in Paris in June 2017, an international consensus [4]. The treatment is symptomatic and should always be related
(ICON) Round Table joining six experts of MD from different conti- to the main complain of the patient. It must be conservative in
nents (Asia, America, Europe, and Australia) was designed in trying the first place. The conservative treatments are used whatever
to draw a minimal consensus, which could be summarized in an the hearing function, as destructive ones are preferentially used
in patients with hearing loss. Concerning bilateral MD, the dif-
ficulty is the unilateral presentation at the beginning and the
∗ Corresponding author. Otology and skull base, Bicêtre universitary hospital, delay of the contralateral involvement. That is why the treat-
AP–HP, 78, rue du Général-Leclerc, 94270 Paris, France. ment should always be conservative. Caregivers must remember
E-mail address: (J. Nevoux). the natural evolution of MD, especially the resolution of vertigo,
1879-7296/© 2018 Elsevier Masson SAS. All rights reserved.

Please cite this article in press as: Nevoux J, et al. International consensus (ICON) on treatment of Ménière’s disease. European Annals
of Otorhinolaryngology, Head and Neck diseases (2017),
G Model
ANORL-731; No. of Pages 4 ARTICLE IN PRESS
2 J. Nevoux et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx

and the major implication of the placebo effect in any kind of symptoms, especially vertigo [20]. To manage conservatively the
treatment. rest of the patients, intratympanic injection of steroids (ITS) is pro-
Finally, the discussion before and during the congress between posed as a second-line treatment. It is more and more popular [21].
participants of ICON Round Table has led to propose a minimal Dexamethasone [22] is more used than methylprednisolone [23]
intercontinental consensus on MD treatment, reported here. It (Level of proof 2 for both). Patel et al. recently reported that two
reviews the different therapeutic options for the unilateral form, injections of methylprednisolone (62.5 mg/mL) given 2 weeks apart
makes a focus on the bilateral form, and proposes a treatment was safe and as efficient as gentamicin (40 mg/mL) used with the
algorithm based on a review of the literature and the authors expe- same protocol to treat refractory MD. Most authors use daily injec-
rience. tions of dexamethasone solution (4 mg/mL) for five consecutive
days. For the authors, the use of one injection per week for 1 to 4
consecutive weeks is also efficient [24]. ITS significantly improves
2. First step: medical treatment of Ménière’s disease
both frequency and severity of vertigo spells compared to placebo
at 24 months after treatment (Level of proof 2). The authors rec-
The authors recommend (Grade C), as the first care given to the
ommend the use of ITS, whatever the drug, preferably with the
patient, the modification of the lifestyle including well sleeping [5]
protocol previously described to treat patients with MD in a non
(Level of proof 4), and a research of an obstructive sleep apnea
ablative manner (Grade B) since these drugs are not ototoxic [25]
syndrome [6] (Level of proof 2), decreasing stress, avoiding caf-
(Level of proof 1).
feine, alcohol and tobacco [7] (Level of proof 4) and adopt a low
salt diet. Two treatment options should be considered to help the
patient: vestibular rehabilitation and psychotherapy [8–10] (Level 4. Third step: surgical conservative treatment
of proof 2). The vestibular rehabilitation should be avoided during
crises and preferentially used in intercritic period. The authors rec- A literature review considering the studies published during the
ommend this rehabilitation (Grade B) even if a recent Cochrane last decade confirms a decline of surgical treatment of MD in favor
review of the literature, using the risk of bias tool, cannot con- of intratympanic injections [20]. The most favored surgical tech-
clude on a positive effect of vestibular rehabilitation on balance nique remains endolymphatic sac surgery (ELSS). It represents for
and dizziness-related quality of life [8]. But this review studied the authors one of the third-line treatments of MD, even if it has
the effects of vestibular rehabilitation in all unilateral peripheral long been criticized and considered as a placebo surgery. Most crit-
vestibular dysfunctions, including MD. In this context, it reported ics referred on two placebo-controlled Danish studies analyzed in
moderate to strong evidence that vestibular rehabilitation was a the Cochrane review in 2010 and 2013. Both studies concluded that
safe and effective management [9] (Level of proof 2). Considering ELSS has no evidence-based effect on natural course and vertigo
psychotherapy, especially the cognitive behavior therapy inter- of MD [26,27] (Level of proof 2). But a more recent meta-analysis
vention, it produces significant improvements in dizziness-related came to the conclusion there was a low level of evidence in favor
symptoms, disability, and functional impairment among patients of an effect [28] (Level of proof 2). This controversy is due to the
with chronic subjective dizziness [10] (Level of proof 2). great difficulty to evaluate in a blinded way surgical treatments in
Diuretics represent the most commonly used first-line medical MD, since the choice of a placebo or control reveals rather impossi-
treatment. The drug chosen differs from one author to another but, ble. For most authors today, grommet insertion or mastoidectomy,
according to the literature, hydrochlorothiazide, acetazolamide and respectively chosen in the Danish studies, cannot be considered
chlorthalidone are used in decreasing order [11,12] (Level of proof as placebo treatments. Therefore the conclusion of these studies is
4). The authors recommend their use that may decrease vertigo not relevant. This is underlined in the Cochrane review. Even if solid
spell frequency (Grade C). A special attention must be payed to their proofs are lacking in the literature, the authors agree that it should
respective contraindications and side-effects. be the first option after failure of the medical conservative treat-
Betahistine is very popular in France, Europe, Japan and with ment, if hearing function useful and MD in young subjects (Grade
many surgeons in Australia but not FDA-approved in USA. The B). All the authors favour ELSS but the evolution of the practice is
dosage varies from one center to another but the literature reports toward an increase number of ITS, especially in USA and in France,
a better effect with a minimal dosage of 48 mg/d. It could be used up and a decrease number of ELSS surgeries.
to 288 to 480 mg/d for patient with severe MD who does not suffi-
ciently respond to lower dosages [1–17] (Level of proof 1 and 3). The
5. Fourth step: medical destructive treatment of Ménière’s
authors recommend, depending on their country, using betahis-
tine at a dose of 48 to 96 mg/d (Grade C). Even if the side effects for
higher dosage seem rare and the efficacy better in some reports, the
Intratympanic injection of gentamicin (ITG) is probably the most
authors do not give recommendation for these dosages for security
effective non-surgical treatment to eradicate vertigo in MD. But it
® is also an ablative method that carries a non-negligible risk of hear-
Another non-invasive option is the Meniett system (Medtronic
ing loss [29] (Level of proof 2). Currently, ITG is favored in USA and
Cie, the USA), producing sequences of micro-pressure pulses sus-
most European countries. But it is about to change, as in France,
ceptible to act on the endolymphatic hydrops [18]. This device is
Japan and Australia, ITS is preferred to ITG as a second-line treat-
included of the algorithm treatment in Italy and Australia, even if
ment. Concerning ITG, no consensus has been reached so far on the
a recent Cochrane review concludes there is no evidence this ther-
dosage and treatment duration as Syed et al. has reported recently
apy is effective [19] (Level of proof 2). Because of the very low rate
in a review of the literature [24]. The authors recommend using
of side effects reported in the five studies of the Cochrane review,
ITG as a destructive method preferentially when hearing function
the authors recommend using this device as a first line treatment
is impaired for patients with good contralateral vestibular func-
(professional agreement).
tion (Grade A). Based on Syed et al. meta-analysis, is advocated a
“titration” protocol, ITG injections (40 mg/mL) being repeated until
3. Second step: intratympanic corticosteroids disappearance of vertigo spells. This “tailored” protocol is about
to prevent hearing loss more than systematic weekly or monthly
At that point, after using one or all of these therapeutic options, injection [24,30] (Level of proof 2 and 4). As a systematic genetic
the authors confirm that 80% of patients are in remission of MD screening of MD patients is not currently done, hypersensitivity

Please cite this article in press as: Nevoux J, et al. International consensus (ICON) on treatment of Ménière’s disease. European Annals
of Otorhinolaryngology, Head and Neck diseases (2017),
G Model
ANORL-731; No. of Pages 4 ARTICLE IN PRESS
J. Nevoux et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx 3

Fig. 1. Proposition of algorithm of treatment of Ménière’s disease.

to the aminoglycosides in carriers of the mitochondrial mutation 8. Proposition of algorithm of MD treatment

of the gene MTRNR1 is not screened. This mutation exposes to a
complete and definitive deafness after a single injection of amino- The Fig. 1 represents a proposal of an algorithm of MD treatment
glycosides [31]. ITG is now in transition between the third and the as an international consensus obtains for the IFOS meeting 2017.
fourth line of treatment of the MD. The first line of treatment includes the medical conservative treat-
ment. After this line of treatment 80% of patients with MD are cured
or in remission. Then the second line is the IT injections, mainly ITS
6. Fifth step: surgical destructive treatments as a conservative treatment and ITG in case of failure and preferen-
tially in patients with hearing impairment. After this second line, 90
Evidence-based evaluations of totally ablative techniques, rep- to 95% of the patients are cured or in remission [36]. The third line
resented by surgical labyrinthectomy and vestibular neurectomy is the surgical, conservative or destructive, treatment. If indicated,
(VN) are scarce in the literature, compared to that of ELSS. No ELSS must be indicated before ITG.
randomized controlled trials are available in the literature. Nev-
ertheless, studies recommend their use after medical treatment
Disclosure of interest
failure and report a very good efficacy to control vertigo in MD
patients. VN reveals more efficient than ITG [32,33]. The authors
The authors declare that they have no competing interest.
recommend indicating VN in patients suffering of intractable ver-
tigo crises not influenced by medical treatment associated with
poor but serviceable hearing function for patients with good con- References
tralateral vestibular function (Grade B). There is a trend to replace
[1] Committee on hearing equilibrium guidelines for the diagnosis evaluation on
ELSS by VN in patients that do not respond to ITG or suffer of drop- therapy in Ménière’s disease. Otolaryngol Head Neck Surg 1995;113:181–5.
attack crises. Surgical labyrinthectomy is less and less performed, [2] Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, et al. Diagnostic criteria for
even if the efficacy is close to the VN, since it totally destroys the Ménière’s disease. J vest Res 2015;25:1–7.
[3] Nevoux J, Franco-Vidal V, Bouccara D, Parietti-Winkler C, et al. Groupe de tra-
remaining hearing function, at the difference with VN [34]. Most
vail de la SFORL. Diagnostic and therapeutic strategy in Ménière’s disease.
authors recommend to associate cochlear implantation to surgical Guidelines of the French Otorhinolaryngology-Head and Neck Surgery Society
labyrinthectomy in order to rehabilitate hearing in the same time. (SFORL). Eur Ann Otorhinolaryngol Head Neck Dis 2017;16:30222–8.
[4] van Esch BF, van der Zaag-Loonen HJ, Bruintjes TD, van Benthem PP. Interven-
This practice is more common in Australia and USA than in Europe
tions for Ménière’s disease: protocol for an umbrella systematic review and a
or Japan. It is not allowed in France. network meta-analysis. BMJ Open 2016;6:e010269.
[5] Holgers K-M, Finizia C. Health profile for patients with Ménière’s disease. Noise
Health 2001;4:71–80.
7. Treatment of bilateral form of Ménière’s disease [6] Nakayama M, Masuda A, Ando KB, Arima S, et al. A pilot study on the efficacy of
contunous positive airway pressure on the manifestations of Ménière’s disease
in patients with concomitant obstructive sleep apnea syndrome. J Clin Sleep
Specific data concerning occurrence of bilateral MD are not Med 2015;11:1101–7.
available and reports are from 2 to 47%. Bilateralization occurs sec- [7] Luxford E, Berliner KI, Lee J, Luxford WM. Dietary modification as adjunct treat-
ment in Ménière’s disease: patients willingness and ability to comply. Otol
ondarily after several years [35]. Obviously, bilateral MD should be Neurotol 2013;34:1438–43.
treated in a conservative way to avoid bilateral deafferentation or [8] van Esch BF, van der Scheer-Horst ES, van der Zaag-Loonen HJ, Bruintjes TD,
ablative treatments. This occurrence has to be anticipated at the et al. The effect of vestibular rehabilitation in patients with Ménière’s disease.
Otolaryngol Head Neck Surg 2017;156:426–34.
time of treating the first ear, especially when there are some argu-
[9] McDonnell MN, Hillier SL. Vestibular rehabilitation for unilateral peripheral
ments to forecast that the contralateral ear is not spared. This is vestibular dysfunction. Cochrane Database Syst Rev 2015;1:CD005397.
the reason why the conservative treatment has always to be used [10] Edelman S, Mahoney AE, Cremer PD. Cognitive behavior therapy for chronic
subjective dizziness: a randomized, controlled trial. Am J Otolaryngol
for the first line, bringing more and more teams to switch from ITG
to steroids ITS. Concerning the surgical treatment, the best option, [11] Thirlwall AS, Kundu S. Diuretics for Ménière’s disease or syndrome. Cochrane
even if controversial, is the ELSS in case of bilateral MD. Database Syst Rev 2006;3:CD003599.

Please cite this article in press as: Nevoux J, et al. International consensus (ICON) on treatment of Ménière’s disease. European Annals
of Otorhinolaryngology, Head and Neck diseases (2017),
G Model
ANORL-731; No. of Pages 4 ARTICLE IN PRESS
4 J. Nevoux et al. / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2017) xxx–xxx

[12] Crowson MG, Patki A, Tucci DL. A systematic review of diuretics in the randomised, double-blind, comparative effectiveness trial. Lancet 2016;388:
medical management of Ménière’s disease. Otolaryngol Head Neck Surg 2753–62.
2016;154:824–34. [24] Philips JS, Westerberg B. Intratympanic steroids for Ménière’s disease or syn-
[13] James AL, Burton MJ. Betahistine for Ménière’s disease or syndrome. Cochrane drome. Cochrane Database Syst Rev 2011;7:CD008514.
Database Syst Rev 2001;1:CD001873. [25] Syed MI, Ilan O, Nassar J, Rutka JA. Intratympanic therapy in Ménière’s syn-
[14] Strupp M, Zwergal A, Feil K, Bremova T, Brandt T. Pharmacotherapy of vestibular drome or disease: up to date evidence for clinical practice. Clin Otolaryngol
and cerebellar disorders and downbeat nystagmus: translational and back- 2015;40:682–90.
translational research. Ann N Y Acad Sci 2015;1343:27–36. [26] Pullens B, Giard JL, Verschuur HP, van Benthem PP. Surgery for Ménière’s dis-
[15] Lezius F, Adrion C, Mansmann U, Jahn K, Strupp M. High-dosage Betahis- ease. Cochrane Database Syst Rev 2010;1:CD005395.
tine dihydrochloride between 288 and 480 mg/day in patients with severe [27] Pullens B, Verschuur HP, van Benthem PP. Surgery for Ménière’s disease.
Ménière’s disease: a case series. Eur Arch Otorhinolaryngol 2011;268:1237–40. Cochrane Database Syst Rev 2013;2:CD005395.
[16] Albu S, Chirtes F, Trombitas V, Nagy A, Marceanu L, Babighian G, et al. Intratym- [28] Lim MY, Zhang M, Yuen HW, Leong JL. Current evidence for endolymphatic sac
panic dexamethasone versus high dosage of betahistine in the treatment of surgery in the treatment of Ménière’s disease: a systematic review. Singapore
intractable unilateral Ménière disease. Am J Otolaryngol 2015;36:205–9. Med J 2015;56:593–8.
[17] Adrion C, Fischer CS, Wagner J, Gürkov R, et al. Efficacy and safety of betahistine [29] Pullens B, van Benthem PP. Intratympanic gentamicin for Ménière’s disease or
treatment in patients with Ménière’s disease: primary results of a long term, syndrome. Cochrane Database Syst Rev 2011;3:CD008234.
multicentre, double blind, randomised, placebo controlled, dose defining trial [30] Quaglieri S, Gatti O, Rebecchi E, Manfrin M, et al. Intratympanic gentamicin
(BEMED trial). BMJ 2016;352:h6816. treatment “as needed” for Ménière’s disease. Long-term analysis using the
[18] Covelli E, Volpini L, Atturo F, Benincasa AT, et al. Delayed effect of active pressure Kaplan-Meier method. Eur Arch Otorhinolaryngol 2014;271:1443–9.
treatment on endolymphatic hydrops. Audiol Neurootol 2017;22(1):24–9. [31] Prezant TR, Agapian JV, Bohlman MC, Bu X, et al. Mitochondrial ribosomal RNA
[19] van Sonsbeek S, Pullens B, van Benthem PP. Positive pressure therapy for mutation associated with both antibiotic-induced and non-syndromic deaf-
Ménière’s disease or syndrome. Cochrane Database Syst Rev 2015;3:CD008419. ness. Nat Genet 1993;4:289–94.
[20] Claes J, Van de Heyning PH. A review of medical treatment for Ménière’s disease. [32] Silverstein H, Wanamaker H, Flanzer J, Rosenberg S. Vestibular neurectomy in
Acta Otolaryngol Suppl 2000;544:34–9. the United States 1990. Am J Otol 1992;13:23–30.
[21] Syed MI, Iian O, Leong AC, Pothier DD, et al. Ménière’s syndrome or disease: tie [33] Morel N, Dumas G, Nguyen DQ, Mohr E, et al. Vestibular neurotomy versus
trends in management and quality of evidence over the last two decades. Otol chemical labyrinthectomy for disabling Ménière disease. Ann Otolaryngol Chir
Neurotol 2015;36:1309–16. Cervicofac 2005;122:271–80.
[22] Garduno-Anaya MA, Couthino De Toledo H, Hinojosa-Gonzalez R, Pane-Pianese [34] De La Cruz A, Borne Teufert K, Berliner K. Transmastoid labyrinthectomy versus
C, et al. Dexamethasone inner ear perfusion by intratympanic injection translabyrinthine vestibular nerve section: does cutting the vestibular nerve
in unilateral Ménière’s disease: a two-year prospective, placebo-controlled, make a difference in outcome? Otol Neurotol 2007;28:801–8.
double-blind, randomized trial. Otolaryngol Head Neck Surg 2005;133: [35] House JW, Doherty JK, Fisher LM, Derebery MJ, et al. Ménière’s disease: preva-
285–94. lence of contralateral ear involvement. Otol Neurotol 2006;27:355–61.
[23] Patel M, Agarwal K, Arshad Q, Hariri M, et al. Intratympanic methylpred- [36] Espinosa-Sanchez JM, Lopez-Escamez JA. Ménière’s disease. Handb Clin Neurol
nisolone versus gentamicin in patients with unilateral Ménière’s disease: a 2016;137:257–77.

Please cite this article in press as: Nevoux J, et al. International consensus (ICON) on treatment of Ménière’s disease. European Annals
of Otorhinolaryngology, Head and Neck diseases (2017),